From the Guidelines
Calciphylaxis management should prioritize a multimodal approach, including advanced care planning, pain control, shared decision-making, and dialysis treatment options, as well as considering nonpharmacotherapy interventions such as wound care, as suggested by the most recent KDIGO controversies conference 1. The condition is characterized by calcium deposits in small blood vessels of the skin and subcutaneous fat, leading to painful skin lesions, tissue death, and potentially life-threatening infections. It primarily affects patients with end-stage kidney disease on dialysis, though it can occasionally occur in patients with normal kidney function. Some key aspects of management include:
- Discontinuing medications that promote calcification, such as vitamin D and calcium-based phosphate binders
- Limiting exposure to excess calcium and phosphate
- Avoiding the use of vitamin K antagonists for anticoagulation in patients undergoing dialysis, as they are associated with an up to 11-fold increased risk of developing calciphylaxis 1
- Considering the use of reduced-dose non–vitamin K oral anticoagulants, such as apixaban, as a safe and effective alternative to warfarin in patients with kidney failure on dialysis who have atrial fibrillation with calciphylaxis 1
- Focusing on wound care and pain control, as these are crucial aspects of managing calciphylaxis
- Surgical debridement of necrotic tissue may be necessary, along with appropriate antibiotics for infections The condition occurs due to an imbalance in calcium-phosphate metabolism, with vascular calcification leading to tissue ischemia and necrosis. Mortality rates are high, often exceeding 50% within one year of diagnosis, primarily due to sepsis from infected wounds, highlighting the importance of early recognition and aggressive management.
From the Research
Definition and Prevalence of Calciphylaxis
- Calciphylaxis is a rare but life-threatening condition, most commonly affecting patients with stage 4 or 5 chronic kidney disease 2.
- The prevalence of calciphylaxis in hemodialysis patients ranges between 1 and 4%, while mortality amounts to 30-80% 3.
Risk Factors for Calciphylaxis
- Elevated calcium and phosphate concentrations and hyperparathyroidism are risk factors for calciphylaxis 2, 4.
- Other risk factors include chronic kidney failure, dialysis, and uremia 5.
Treatment of Calciphylaxis
- No universally accepted therapy exists for calciphylaxis, but several noninvasive treatments have been trialled with variable success 2.
- Cinacalcet, a calcimimetic agent, has been shown to be effective in treating calciphylaxis, with a high response rate (83.4% and 82.8% for monotherapy and combination therapy, respectively) 2.
- Sodium thiosulfate, bisphosphonates, and non-calcium-containing binders are also used to target calcification 4, 3.
- A multifactorial approach to controlling postoperative pain is recommended, including the use of nerve blocks, renal-sparing opioids, benzodiazepines, and/or ketamine 6.
Management of Calciphylaxis
- Preoperative and intraoperative management involves monitoring and optimizing patient comorbid conditions and any possible electrolyte imbalances 6.
- Postoperative management can be challenging, with potential triggers such as warfarin and corticosteroids, and poor wound healing and difficult pain control 6.
- A multidisciplinary and multimodal approach is recommended for the treatment of calciphylaxis, including wound care, antibiotics, analgesics, and strategies targeting calcification 3.